CAJ | 학술논문

目的明确视黄酸诱导基因-Ⅰ (RIG-Ⅰ)在特发性炎症性肌病(IIMs)中的表达特点,并探讨其在IIMs免疫病理机制中的作用.方法回顾性选取2010年2月至2014年4月期间在山东大学齐鲁医院行肌活检检查的20例皮肌炎(DM)和20例多发性肌炎(PM)患者的肌肉活检标本,同时选取4例面肩肱型肌营养不良(FSHD)和4例正常肌组织作为对照.所有肌肉标本均进行HE染色及RIG-Ⅰ免疫组织化学染色.应用Western印迹定量检测RIG-Ⅰ的表达含量,并用双重免疫荧光方法检测DM/PM肌肉标本中RIG-Ⅰ与浆细胞样树突状细胞(pDCs)的特异性表面标记分子CD303的共表达.结果 RIG-Ⅰ在正常肌肉组织中无阳性表达,而在DM和PM中可见明显阳性表达,以炎症细胞、血管内皮细胞的特异性阳性表达以及坏死和再生肌纤维胞质处的非特异性弱阳性表达为主,另外在伴有束周萎缩的DM中尚可见RIG-Ⅰ在束周肌纤维胞质中的强阳性表达,而在FSHD中则仅见坏死和再生肌纤维胞质处RIG-Ⅰ的非特异性表达.在DM/PM肌肉标本中,未检测到RIG-Ⅰ与CD303的共表达.与对照组相比(包括FSHD和正常对照)DM/PM组肌肉组织中的RIG-Ⅰ表达含量显著上调(平均灰度值:正常对照组0.003±0.003;FSHD组0.019±0.013;DM组0.901±0.470;PM组0.630±0.444),两组之间差异有统计学意义(P＜0.05).结论 RIG-Ⅰ可能参与了调节Th1细胞因子诱导的趋化因子表达,表明其在一定程度上参与了IIMs免疫病理机制过程,但其在Ⅰ型干扰素介导的固有免疫病理机制中可能不起主要作用.
목적 명학시황산유도기인-Ⅰ (RIG-Ⅰ)재특발성염증성기병(IIMs)중적표체특점,병탐토기재IIMs면역병리궤제중적작용.방법 회고성선취2010년2월지2014년4월기간재산동대학제로의원행기활검검사적20례피기염(DM)화20례다발성기염(PM)환자적기육활검표본,동시선취4례면견굉형기영양불량(FSHD)화4례정상기조직작위대조.소유기육표본균진행HE염색급RIG-Ⅰ면역조직화학염색.응용Western인적정량검측RIG-Ⅰ적표체함량,병용쌍중면역형광방법검측DM/PM기육표본중RIG-Ⅰ여장세포양수돌상세포(pDCs)적특이성표면표기분자CD303적공표체.결과 RIG-Ⅰ재정상기육조직중무양성표체,이재DM화PM중가견명현양성표체,이염증세포、혈관내피세포적특이성양성표체이급배사화재생기섬유포질처적비특이성약양성표체위주,령외재반유속주위축적DM중상가견RIG-Ⅰ재속주기섬유포질중적강양성표체,이재FSHD중칙부견배사화재생기섬유포질처RIG-Ⅰ적비특이성표체.재DM/PM기육표본중,미검측도RIG-Ⅰ여CD303적공표체.여대조조상비(포괄FSHD화정상대조)DM/PM조기육조직중적RIG-Ⅰ표체함량현저상조(평균회도치:정상대조조0.003±0.003;FSHD조0.019±0.013;DM조0.901±0.470;PM조0.630±0.444),량조지간차이유통계학의의(P＜0.05).결론 RIG-Ⅰ가능삼여료조절Th1세포인자유도적추화인자표체,표명기재일정정도상삼여료IIMs면역병리궤제과정,단기재Ⅰ형간우소개도적고유면역병리궤제중가능불기주요작용.
Objective To investigate the expression of retinoic acid-Ⅰ inducible gene Ⅰ (RIG-Ⅰ) in the muscle tissues from patients with idiopathic inflammatory myopathies (IIMs),and to speculate the possible role of RIG-Ⅰ in the immunopathogenesis of IIMs.Methods Muscle specimens were obtained from 20 dermatomyositis (DM) and 20 polymyositis (PM) patients who underwent muscle biopsies from February 2010 to April 2014 at Qilu hospital affiliated to Shandong University.Besides,4 facioscapulohumeral muscular dystrophy (FSHD),and 4 non-myopathic patients were taken as control group.All the biopsy specimens were processed with hematoxylin-eosin and immunohistochemical (Mouse anti human RIG-Ⅰ antibodies) staining.We also examined the co-localization of RIG-Ⅰ and CD303,which is the specific surface marker of plasmacytoid dendridic cells (pDCs),by means of double immunofluorescence staining.Western blot was performed for quantitative analysis.Results There was strong expression of RIG-Ⅰ protein in DM/PM muscle tissues while in normal controls was virtually absent.RIG-Ⅰ was specifically expressed in inflammatory cells and vessel endothelium,and nonspecifically expressed in regenerating and necrotic fibers.Besides,strong positive expression was observed in the perimysial perifascicular fibers of DM.In FSHD muscle tissues,only a few regenerating and necrotic fibers was stained nonspecifically for RIG-Ⅰ.However,co-expression of RIG-Ⅰ and CD303 was not detected in DM/PM muscles.The mean grey value of RIG-Ⅰ observed in DM (0.901 ± 0.470) and PM (0.630 ± 0.444) group was significantly higher than in control group including Normal (0.003 ±0.003) and FSHD (0.019 ± 0.013) groups (P ＜ 0.05).Conclusions RIG-Ⅰ may operate as a mediator in Th1 cytokine-Ⅰ induced chemokine expression,so it is involved in the pathogenesis of IIMs.But RIG-Ⅰ may not play a major role in innate immune reaction mediated by type Ⅰ interferon.